Abstract
BackgroundIntra-abdominal desmoid tumors are rare soft tissue tumors that arise mainly in the mesentery and pelvis. Causes include mutations in the APC gene, which is the causative gene of familial adenomatous polyposis (FAP), estrogen-associated changes after childbirth, and mechanical factors, such as history of abdominal surgery. However, there are cases of intra-abdominal desmoid tumors that develop in the absence of such causes, although they are rare, and diagnosis is often difficult from clinical findings. We encountered 2 cases of patients with sporadic intra-abdominal desmoid tumors with a very unusual onset.Case presentationThe first patient was a 51-year-old man. He presented to the clinic with sudden onset of abdominal pain, and was referred to our department because of a giant tumor detected on abdominal ultrasonography. Imaging diagnosis displayed a 19-cm tumor with internal tumoral hemorrhage, but no definitive diagnosis was made. Resection of the tumor was performed for diagnostic and therapeutic purposes. The second patient was a 41-year-old man. Right hydronephrosis was detected on abdominal ultrasonography at a medical check-up. We diagnosed invasion of the primary mesenteric tumor into the right ureter by diagnostic imaging, and performed ileocecal resection with partial right ureteral resection for a definitive diagnosis and therapeutic purposes. The tumors of both patients had developed from the ileal mesentery. Although the tumors were substantially different in their macroscopic morphology and progression pattern, they showed similar pathological characteristics, i.e., they consisted of bundles of collagen fibrils of spindle-shaped fibroblasts with low cell atypia, and were diagnosed to be desmoid tumors by positive immunohistochemical staining for β-catenin. Both patients had no susceptibility factors for desmoid tumors.ConclusionsTo our knowledge, there have been very few reports to date of sporadic intra-abdominal desmoid tumors that were diagnosed owing to acute abdominal pain caused by tumoral hemorrhage or asymptomatic ureteral obstruction. Intra-abdominal desmoid tumors can cause various symptoms depending on the location of their occurrence and their progression pattern and are often difficult to differentiate from other mesenchymal tumors on imaging findings. Diagnosis therefore requires experience and knowledge that is not bound by preconceptions.
Y Shaped Colonic Duplication Mimicking Intestinal Volvulus: A Case Report and Review of Literature
